{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T05:29:54Z","timestamp":1784698194115,"version":"3.55.0"},"reference-count":91,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Respir Crit Care Med"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Immunodeficiency represents a vast number of diseases and syndromes. Both primary and secondary forms of immunodeficiency are important contributors to the development of bronchiectasis. Primary immune deficiencies, in particular, are increasingly identified and defined as contributors. Specific immune deficiencies that are closely associated with bronchiectasis and as discussed in this article are common variable immunodeficiency, specific antibody deficiency, immunodeficiencies involving immunoglobulin E, DOCK8 immunodeficiency, phosphoglucomutase 3 deficiency, activated phosphoinositide 3-kinase delta syndrome, and X-linked agammaglobulinemia. Each of these primary immune deficiencies has unique nuances. Vigilance for these unique signs and symptoms is likely to improve recognition of specific immunodeficiency in the idiopathic bronchiectasis patient. Secondary forms of immunodeficiency occur as a result of a separate disease process. Graft versus host disease, malignancy, and human immunodeficiency virus are three classic examples discussed in this article. An awareness of the potential for these disease settings to lead to bronchiectasis is necessary to optimize patient care. With understanding and mindfulness toward the intricate relationship between bronchiectasis and immunodeficiency, there is an opportunity to elucidate pathophysiologic underpinnings between these two syndromes.<\/jats:p>","DOI":"10.1055\/s-0041-1730893","type":"journal-article","created":{"date-parts":[[2021,7,14]],"date-time":"2021-07-14T22:34:05Z","timestamp":1626302045000},"page":"525-536","source":"Crossref","is-referenced-by-count":14,"title":["Common Variable Immunodeficiency and Other Immunodeficiency Syndromes in Bronchiectasis"],"prefix":"10.1055","volume":"42","author":[{"given":"Pamela J.","family":"McShane","sequence":"additional","affiliation":[{"name":"Section of Pulmonary and Critical Care Medicine, University of Texas Health Science Center at Tyler, Tyler, Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2021,7,14]]},"reference":[{"key":"ref1","first-page":"6","article-title":"Inflammation: a two-edged sword--the model of bronchiectasis","volume":"147","author":"P J Cole","year":"1986","journal-title":"Eur J Respir Dis Suppl"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1016\/S0140-6736(18)31767-7","article-title":"Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity","volume":"392","author":"P A Flume","year":"2018","journal-title":"Lancet"},{"issue":"03","key":"ref3","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1183\/13993003.00629-2017","article-title":"European Respiratory Society guidelines for the management of adult bronchiectasis","volume":"50","author":"E Polverino","year":"2017","journal-title":"Eur Respir J"},{"issue":"03","key":"ref4","doi-asserted-by":"crossref","first-page":"130","DOI":"10.5694\/mjac14.00287","article-title":"Chronic suppurative lung disease and bronchiectasis in children and adults in Australia and New Zealand Thoracic Society of Australia and New Zealand guidelines","volume":"202","author":"A B Chang","year":"2015","journal-title":"Med J Aust"},{"issue":"S 01","key":"ref5","doi-asserted-by":"crossref","first-page":"i1","DOI":"10.1136\/thx.2010.136119","article-title":"British Thoracic Society guideline for non-CF bronchiectasis","volume":"65","author":"M C Pasteur","year":"2010","journal-title":"Thorax"},{"issue":"01","key":"ref6","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1007\/s10875-019-00737-x","article-title":"Human inborn errors of immunity: 2019 update on the classification from the International Union of Immunological Societies Expert Committee","volume":"40","author":"S G Tangye","year":"2020","journal-title":"J Clin Immunol"},{"issue":"04","key":"ref7","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1007\/s10875-014-0028-1","article-title":"USIDNET: a strategy to build a community of clinical immunologists","volume":"34","author":"K E Sullivan","year":"2014","journal-title":"J Clin Immunol"},{"issue":"06","key":"ref8","doi-asserted-by":"crossref","first-page":"1763","DOI":"10.1016\/j.jaip.2019.02.004","article-title":"The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity","volume":"7","author":"M G Seidel","year":"2019","journal-title":"J Allergy Clin Immunol Pract"},{"issue":"18","key":"ref9","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1056\/NEJM197305032881814","article-title":"Classification of primary immunodeficiencies","volume":"288","author":"M D Cooper","year":"1973","journal-title":"N Engl J Med"},{"issue":"05","key":"ref10","first-page":"927","article-title":"Primary immunodeficiencies. 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